Abstract

Rationale

Right ventricular (RV) failure is the main mortality determinant in idiopathic pulmonary arterial hypertension (iPAH). However, little is known about adaptive and maladaptive changes in RV performance during disease progression. This study aims to characterize global RV function in mild, intermediate and severe iPAH based on patient survival duration from iPAH diagnosis.

Methods

Right heart catheterization and MRI were performed in 67 individuals (Con=10, Mild=28, Intermediate=11, Severe=18). iPAH severity was defined as: less than 6 months survival (severe), between 1-3 years survival (intermediate) and more than 5 years survival (mild). RV systolic elastance (Ees), arterial elastance (Ea), RV coupling (Ees/Ea) and diastolic elastance (Ed) were quantified by pressure-volume analysis.